POS0016 COMMON VARIABLE IMMUNODEFICIENCY DISEASE FROM THE PERSPECTIVE OF RHEUMATOLOGY
Authors: T. Yuce Inel, G. Can
Journal: Annals of the Rheumatic Diseases (2022)

Background Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by impaired B cell differentiation and immunoglobulin production. In addition to increased susceptibility to infection, patients also have an increased tendency to autoimmune disease. Since rheumatological findings usually start earlier than other autoimmunities, it is very important to increase the awareness of rheumatologists about this disease, for accurate diagnosis and to prevent delay in treatment. Objectives To increase the awareness of rheumatologists about the main symptoms and findings of CVID. Methods Adult patients referred to the rheumatology department since January 2015 were included in the study. Demographic and clinical characteristics (infections, pulmonary and extrapulmonary granulomatous involvement, autoimmune manifestations), laboratory and imaging findings and treatments of the patients were analyzed. Results Ten adult patients with CVID were included in the study. The gender distribution of the patients was similar and the median age was 38±10.0 years. The mean duration of diagnosis was 123.5±89.3 months. At least one autoimmune manifestation was observed in 80% of the patients. In the follow-up period, 40% of the patients developed arthritis. Involvement of lower extremity joints such as knee and ankle was more prominent. While all patients were given 0.8 g/kg/3 weeks of intravenous immunoglobulin, 80% required immunosuppressive therapy for autoimmune manifestations. The demographic and clinical characteristics of the patients are summarized in Table 1. Table 1. Autoimmune manifestations of common variable immunodeficiency patients Case 1 Case 2 Case 3 Case 4 Case 5 Case 6 Case 7 Case 8 Case 9 Case 10 Age/Sex 36/F 43/F 40/M 25/M 23/M 36/M 41/M 57/F 35/F 48/F Symptom duration (years ) 12 11 11 4 17 12 11 13 12 7 Diagnosis duration (years ) 6 11 8 3 17 10 9 1 11 2 Sinopulmonary infection + + + + + + + + + + GI infection - + - + - + + - - - Other infections + + - + + - + + - + Pulmonary Involvement lymphocytic ILD nodule *bronchiectasis - - bronchiectasis bronchiectasiscavitation nodule nodule - Extrapulmonary Granuloma brain parenchyma, bone marrow, liver, spleen, skin liver, spleen - renal°, liver - **bone - ᵜlymph node - - Autoimmune manifestations - monoarthritis,chronic ileitis oligoarthritis,atrophic gastritis,chronic ileitis alopecia, thyroiditis,bilateral anterior uveitis - oligoarthritis,autoimmune neutropenia,chronic ileitis,kounis syndrome oligoarthritis,autoimmune neutropenia oligoarthritis, thyroiditis,sicca syndrome,recurrent scleritis colitis - Cirrhosis of the liver + + - - - + - - - - Lymphadenopathy + + + + - + + + + + Splenomegaly + + + + - + + - + - Immunosuppressive Treatment Steroid, AZA, CSA, Adalimumab Steroid Steroid, SSZ Steroid, MMF, CYC - Steroid, SSZ, MTX, LEF, CSA Steroid Steroid, MTX, AZA, CSA Steroid, AZA - Abbreviations: AZA-azathioprine, CSA-cyclosporine, CYC-cyclophosphamide, F-female, GI-gastrointestinal, ILD-interstitial lung disease, LEF-leflunomide, M-male, MTX-methotrexate, SSZ-sulfasalazine *right lower lobectomy, ** right foot 5th toe enucleation, ᵜ mediastinoscopic lymph node biopsy ° interstitial granulomatous nephritis Conclusion Autoimmune diseases can be seen in patients with CVID, and sometimes this may be the first presentation of CVID. Heterogeneous clinical findings of the disease may lead to delay in diagnosis. Clinicians should be more careful about the different manifestations of CVID to avoid delay in diagnosis. Disclosure of Interests None declared